Hematology 2 Flashcards

Terms Definitions
vitamin B12 is aka:
Where is iron absorbed?
Normal Reticulocyte Count for Adults:
Internal or external bleed?

iron recaptured
Qualitative defect in hemoglobin:
Sickle Cell Anemia
Cooperativity: as you bind more oxygen, the ability to bind more oxygen increases, so 4 sites for oxygen to bind on HGB and affinity for O2 increases with each binding of O2 molecule
How to diagnose immunohemolytic anemia?
Coombs test
Cold or warm immunolytic anemia?

- mycoplasma
- infectious mono
- influenza
Which drug would cause megaloblastic anemia, specifically folate deficiency?
What is this disease?

- hypochromic, microcytic,basophilic stipling
- aniscocytosis, poikilpcytosis
- target cells
- marked increase in HbA2 and HbF
Iron deficiency anemia causes problems with ___ ___. This is the most common anemia worldwide and most common anemia in children.
RBC maturation
Iron deficiency anemia summary:
Microcytic MCV RDW Widened and shifted to leftHypochromic MCH Retic. count May be > 2 %Serum ferritin Very low TIBC Increased > 340 (µg/dL)BM Iron stain Iron is low to Absent Response to Fe Rx. Excellent
What is this disease?

- microcytic, hypochromic
- atrophic glossitis
- esophageal webs
Plummer-Vinson syndrome
What is this disease?

- no spleenomegaly
- reticulocytopenia
- slightly increased MCV
- fibrous stroma in bone marrow
- hypocellular bone marrow
aplastic anemia
What is this disease?

- increase in Fe, ferritin
- decrease in TIBC
What is this disease?

- macrocytic
- normochromatic
- low reticulocyte count
megaloblastic anemia
Is PNH inheritable?
No. The mutation is acquired.
get rid of lead poisoning by chelating with __.
If someone has lead poisoning, what type of anemia do they likely have?
sideroblastic anemia
symptoms of Sickle cell anemia:
- chronic: jaundice, hepatosplenomegaly- acute: triggered by infection and dehydration, bone pain in chest and back, abdominal pain, priapism, stroke
In thalasesemia you have all the iron, but you don’t have all the proteins necessary to bind oxygen to its best capability
continue iron supplement for at least __ __ after normal Hb
2 months
primary iron storage protein in body. Serum ferritin is an indirect reflection of total iron stores. Free iron is toxic due to catalyzation of hydrogen peroxide into free radicals so binding to ferritin is essential.
What is Fanconi anemia?
defect in multiprotein complex required for DNA repair -> T cell activation -> IFN,TNF -> bone marrow suppression
What is this disease?

- normal Fe+
- increase ferritin
- decrease in TIBC
beta-thalassemia trait
What is this disease?

- teat drop RBC
- marrow infiltrated with lymphocytes
myelophthisic anemia
What is this disease?

- hand-foot syndrome
- pain crisis
- pigmented gallstone
- leg ulcer
- vision loss
sickle cell anemia
Clinical manifestations of B12 deficiency:
- Megaloblastic anemia- Pancytopenia- Paresthesias- Peripheral neuropathy- Demyelination of corticospinal and dorsal column tracts- Irritability - Personality changes- Possible increased risk of stroke/MI
Microcytic anemiaNormal Iron studiesLow RBC countWhat are you thinking?
- Sideroblastic anemia- Anemia of chronic disease
Vitamin B12 is needed for __ synthesis.
DNA (and myelin)
An enzyme disorder in which the body has adequate iron but is unable to incorporate it into hemoglobin?
Sideroblastic anemia
MCV between 81 and 100 flIncreased reticulocyte countNo evidence of bleedingIndirect (unconjugated) bilirubinemia
Normocytic hemolytic anemia
What is this disease? be specific.

- morphology of RBC: normal
- painless hematuria
Sickle cell trait
What substances inhibit iron absorption?
- tea
- carbonates
- oxalates
- phosphate
What is this disease?

- normocytic, normochromatic RBC
- low Fe+,transferrin
- high ferritin, high storage in bone marrow macrophages
anemia of chronic disease
What is a congenital pure red cell aplasia?
Blacfan-Diamond syndrome
Which drugs act as folate antagonists?
- methotrexate
- trimethoprim
- pentamadine
Folate deficiency affect which type of DNA synthesis? (purine or pyrimidine)
- DTMP synthesis
Which model of immunohemolytic anemia do the following fit?

- penicillin
- cephalosporin
- quinidine
hapten model (warm antibody)
Which type alpha-thalassemia is completely asymptomatic?
silent carrier state: single alpha-globin gene mutation.
Why are the cells bigger in megaloblastic anemia?
asynchronous nuclear/cytoplasm maturation.
4 causes of Microcytic Anemia:
- Iron Deficiency Anemia- Thalassemia- Sideroblastic Anemia- Anemia of chronic disease
Basic etiology of anemia:
- not making enough- losing too much
Drugs that cause normocytic hemolytic anemia:
- Sulfa agents: nitrofurantoin- Quinidine/quinine
Additional symptoms for iron deficiency anemia:
- PICA- koilonychias (spoon nails)- soreness of the mouth with cracks at corners- dysphagia (Plumber-Vinson Syndrome)
How to treat thalassemia?
- iron chelator for iron overload
- HLA matched bone marrow transplant
What is Donath-Landsteiner antibody?
antibody for P antigen developed after syphillus infection.

It may cause paroxysmal cold hemolgobinuria
Why is MCH elevated in HS?
loss of Na and H2O
What substances inhence iron absorption?
- ascorbic acid
- citrate acid
- sugar
Lab values for folate deficiency macrocytic anemia:
- low folate- normal methylmalonic acid- elevated homocysteine
With Hereditary spherocytosis hemolysis occurs due to trapping of __ __ cells. There will be an __ RDW due to ___ retics and spherocytes.
- stiff rigid cells- increased RDW due to increased spherocytes and retics
Sideroblastic anemia caused by lead tmt:
Lead poisoningLead lines on gingivae and epiphyses of long bones on XrayEncephalopathy and Erythrocyte basophilic stipplingAbdominal colic and microcytic AnemiaDrops: wrist and foot drop. Dimercaprol and EDTA as first line of treatmentRed cell transfusion – Vit B6 to reverse the anemiadesferrioxamine (deferoxamine) - iron chelator
Additional symptoms of anemia caused by chronic RBC destruction (hemolysis):
- Jaundice- Brown/red urine- leg ulcers- failure to thrive in infancy- gallstone symptoms
Clinical signs of anemia upon PE:
- skin/mucosal pallor- skin dryness- bald tongue/glossitis- mouth ulcers/rectal exam- jaundice/purpura- lymphadenopathy- hepato-splenomegaly- breathlessness (RR above 20 is high)- tachycardia, CHF- bleeding, occult blood
What is the underlying cause of pernicious anemia?
antoreactive T cell -> antoantibodies against IF
What are the functions of B12?
- cofactor for conversion of homocysteine to methionine
- aid isomerizaition of methylmalonyl coA to succinyl coA.
How does B12 get to the plasma?
B12 associated with transcobamalineII in the ileum mucosal cell.
What are some complications of PNH?
- episodic venous thrombosis: hepatic, portal, cerevral veins.
- risk of developing AML: because this is mutation in stem cells.
Which drugs block uptake of folate in the jejunum?
- anticonvulsants
What condition may trigger symptoms in HS patients?
- parvovirus infection
- infectious mono
- gallstones
Which types of anemias are intravascular?
- severe G6PD
- mechanical trauma
- acute blood loss (can also be extravascular)
What are some general causes of aplastic anemia?
1) dose dependent myelotoxins
- benzene
- chloramphenicol
- alkylating agents
- antimotabolites

2) idiosyncratic reaction to small dose drugs
- streptomycin
- chloramphenicol
- phenylbutazone
- chlorpromazine

3) whole body irradiation

4) viral infections: viral hepatitis
What are some complications of hemolysis?
- anemia
- aplastic crisis (parvovirus infect stem cells)
- folic acid deficiency(megaloblastic anemia): chronic hemolytic anemia should receive daily folic acid.
- skeletal deformity:"hair on end" appearance on X-ray.
- gallstones: pure bilirubin
- Fe overload (extravascular hemolysis only)
- DIC (intravascular hemolysis only and complement activation)
- Kernicterus: bilirubin deposits in basal ganglia
See patient is anemia, but the size is between 81 and 100 fl. Now what?
Normocytic anemiaNext check reticulocyte count
MCHC measures __ __. The terms __ and __ refer to changes in __ __ in the red cells.
- hemoglobin concentration- hyperchromic and hypochromic - hemoglobin concentration
Sideroblastic anemia requires a __ __ __ for diagnosis. This will show __ __.
- bone marrow assessment- ringed sideroblasts
What is this disease?

- more common in Asian
- moderate anemia
- resemble beta-thalassemia intermedia
HbH: 3 alpha globin gene deletion
What are some triggers of crisis in G6PD?
Oxidative stress
- infections: viral hepatitis, pneumonia, typhoid fever
- drugs: antimalarial, sulfonamide, nitrofurantoins
- fava beans
What is the pathogenesis of HbH?
3 alpha-globin gene deletion:
- high affinity for O2 -> hypoxia
- prone to oxydation
What are some complications of B12 deficient patients?
- atrophic glossitis: shiny, glazed tongue.
- atriphy of fundic gland
- gastric metaplasia, cancer
- neurologic defect: subacute combined degeneration
- atherosclerosis,thrombosis: elevated homocysteine and methylmalonic acid.
What are some causes of B12 deficiency?
- long term inadequet diet
- gastrectomy
- loss of pancreatic function
- ileal resection
- tape worm
- pregnancy, hyperthyroidism, disseminated cancer, chronic infections.
TIBC measures the extent to which iron binding sites in the serum can be __ . It is an indirect measure of ___ and ___ related to ___ saturation.
- saturated - transferrin- inversely related to transferrin saturation
Additional symptoms of B12 deficiency anemia:
- parasthesias - loss of sense of touch- wobbly gait/difficulty walking- clumsiness and stiffness in arms and legs- dementia- hallucinations, paranoia, and schizophrenia
Heinz bodies on a blood smear in an AA male patient?
G6PD normocytic hemolytic anemia
G6PD deficiency is a __ __ disease that affects __ of African American and Meditteranean descent. You will see __ __ on the smear.
- x-linked - males- Heinz bodies
What is the most common genetic defect in PNH?
CD59: potent inhibitor of C3 convertase
What is this disease?

- elevated reticulocytes, indirect bilirubin, RDW
- decreased RBC level, haptoglobulin
- positive osmotic fragility test
- HS: ankyrin mutation
- HE: band 3 protein mutation
What are some mutations involved in PNH?
- CD59: potent inhibitor of C3 convertase
- CD55
- CD8 binding protein
A large RDW suggests large __ in sizes of RBCs. This is called ___. This value is calculated by:
- variability - anisocytoses- RDW= (standard deviation of red cell volume/MCV)x100
If HGB and HCT are low> anemiaIf MCVNow what?
Check for iron deficiency by ordering: - Serum iron- non-heme iron, variability day to day- Ferritin- best for iron stores- Total Iron Binding Capacity- good sensitivity for low iron but low specificity- Fe or Transferrin saturation- serum iron/TIBC
what does a plain CBC include:
- RBC count- WBC count- HGB- HCT- MCV- MCH- MCHC- Platelets
What are some factors for cell sickling? Which is the most important?
- interaction of HbS with other Hgb chains: most important
- MCHC: alpha thalassemia lowers MCHC which lowers the sickling
- low pH: lowers O2 affinity, facilitates sickling
- long transit time: facilitates sickling
- amount of Hb in plasma: inactivates NO, increases vascular tone, facilitates sickling
Is hepcidin level high or low in iron deficiency anemia?

Hepcidin: inhibit iron uptake and release from macrophages
Anemia with MCV b/w 81 and 100 fl. Reticulocyte count is normal or decreased. Whats going on?
- anemia of chronic disease- primary bone marrow problem
Where is folate stored and for how long?
Folate is stored in the liver for months.
lab of pt with beta thalassemia major:
- low hcg and hct- mcv less than 80 fl= microcytic- hypochromic- normal iron levels- normal to high RBC count- low RDW- family history
varied, irregular
abnormal, difficult, bad
Peripheral Blood Smears
Hereditary coagulation disorder
Sex-linked recessive disorder
Von Willebrand’s disease
Hemophilia A (most common)
Hemophilia B
T-ALL immuno markers
"CD2+,3+,7+, TdT+"
Cells store iron as:
Usual mutation in alpha-thalassemia
Essential Thrombocythemia

plts consistently >450K
increased/weird megakaryocytes in BM

Not CML, inflammation, iron deficiency
Normal WBC lab value
HIV Deaths (Per year??)
3 million
Possible Myelodysplastic Tx
"5-azacytidine, decitabine, lenalidomide"
Thalidomide (Thalomid) Use: Multiple myeloma Mechanism: angiogenesis inhibitor, TNF blocking agent
Bohr Effect
increased pH=increased hgb-oxygen affinitydecreased pH=decreased hgb-oxygen affinity
What blood cell?
Kidney shaped nucleus
Pleger-Huet cells
Hyposegement PMNs

Seen in MDS
Immunocompromised Burkitt's
AIDS associated
Abdominal masses, marrow, lymph nodes
30% are EBV positive
Macrocytic anemias can develop from megaloblastic & nonmegaloblastic causes
what is anisocytosis
amts of RBCs
Transfusion complications
hyperkalemia; hypoglycemia- stored blood has high glucose content, rebound insulin rise ; acid-base derangements- metabolism of citrate in CPD leads to late metabolic alkalosis; hypocalcemia and hypomagnesemia- caused by binding of these ions by citrate p
Currently in widespread use to stimulate HbF production in patients with Sickle Cell Disease
4 myeloproliferative disorders
"CML, PV, ET, myelofibrosis"
TTP pathogenesis
deficiency of vWF cleaving mettaloprotease (ADAMTS13)
When do young, healthy individuals typ. begin to experience sx of anemia?
Hemolytic Anemia: Background
Hemolytic Anemia: Background
- Hemolytic anemias are due to shortening of red cell life span (i.e. increased red cell destruction)
- They involve increased red cell destruction with liberation of hemoglobin or its degradation products.
Laboratory findings:
- Increased unconjugated (indirect) bilirubin
- Hemoglobinemia
- Methemalbuminemia (occur if red cell destruction is very rapid and within the circulation (intravascular hemolysis)
Urine findings:
- No bilirubinuria
- Hemolytic anemias result in acholuric jaundice, which is jaundice not accompanied by bilirubinuria.
- Increased urine urobilinogen
Ultrasound findings:
- The resulting hyperbilirubinemia may lead to pigment-containing gallstones  as a late complication.
brs pathology p.160
continue from there
Hypercoagulability states
Thrombocytosis - increased platelets >1,000,000Atherosclerosis Diabetes Smoking Hypercholesterolemia Congestive heart failure Pregnancy Oral contraceptives - estrogen increases hepatic sythesis of coagulatoin factors Postoperative state - immobility, stasis, injury, prolonged bedrest Malignancies - tumor releases factors Myeloproliferative disrders - polyccythemia Post splenectomy Genetic disorders -leiden mutation - factor V mutation, predisposed to venous thrombosis-antiphospholipid syndrom
Shwachman-Diamond Syndrome
Classic Triad: Neutropenia, exocrine pancreatic insufficiency and skeletal abnormalities
↑ apoptosis of bone marrow cells via Fas(pro-apoptotic)
Describe acute monocytic leukemia, well-differentiated in PB.
-monocytoid cells
Sideroblastic anemia smear
Microcytic anemia
Basophilic stipling creating a ring

Dimorphic populations
CML pathogenesis
t(9;22) creates fusion product BCR-ABL

BCR-ABL is an unregulated tyrosine kinase that stimulates proliferation
measure of volume/size; range 80-99 femtaliters; macrocytic= >99; microcytic= <80
Where are neutrophils produced?
In bone marrow
sickle cell anemia
characteristics of auto splenlcetomy
most primitive RBC; large, remain nucleated, and have a greater MCV; gradually replaced by normoblastic erythrocytes as gestation progresses
What is a reticulocyte?
a newly formed erythrocyte
Pathogenesis of Neutropenia
Ineffective Neutrophil Production by the Bone Marrow (drugs - adults, infections - kids, aplastic anemia, marrow replacement); Accelerated Destruction - neutrophils are made at a normal rate/ amount, but they're destroyed peripherally (immune mediated, splenic sequestration); The 2 most common causes of Severe Neutropenia are drugs in adults and infections in kids
S/S of Iron Deficiency
Constitutional sxs (weakness, dizziness); Tachycardia; Decreased exercise tolerance (exertional dyspnea); Pallor
Where are vit K dependent factors made?
Genetic abnormality seen in M1 AML
"t(9,22)- 10-15%"
Improvement of Hb w/ iron supplementation in iron-def. anemia is not seen until 6-8 wks, what test could be performed to monitor efficacy of tx /p only 2 wks?
Reticulocyte count
A hormone that regulates red blood cell production.
maturation time from Rubriblast to Erythrocyte
3-5 days
shift to the right
decreased hgb-oxygen affinityDPG ↑pH ↓temp ↑
An RBC containing an abnormally low concentration of hemoglobin is _______________.
What type of anemia?
B12 deficiency
Macrocytic megaloblastic anemia
calculating MCH
MCH = Hemoglobin X 10/RBC Count
What disease states are associated with cabot rings?
-myelodysplastic syndrome
-megaloblastic anemia
What percentage of pts that survive TTP will relapse within 10 years?
Fibrinolytic bleeding disorders
Typically delay (12-24 hrs later)
Mucocutaneous, GU

Rare hereditary -- quebec plt disorder, alpha-2-antiplasmin, pai-1 deficiency,

More commonly acquired -- cancer, liver disease, DIC, prostate surgery
exploded, abnormal nucleus of a red blood cell
Decrease in neutrophil count as a result of decreased production or increased destruction
Increased risk for infection
Not a disease but a syndrome
What is a decrease in erythrocytes called?
The degree of effective erythropoietic activity in any hematological disorder is most readily assessed by the:
Retic count
Disseminated intravascular coagulation (DIC)- definition
acquired hemorrhagic disease, triggered by underlying condition; uncontrolled activation of coagulation and fibrinolysis (depletion of platelets, porthrombin, firbinogen, factors V, VIII, XIII
What blood product do burn pts need the most of?
Name a common cause of acquired porphyria that works by inhibiting ALA dehydratase and ferrochelatase
Lead poisoning
Kidneys and Lymphoma
Primary renal lymphoma is rare; Secondary involvement is seen late in the disease; Non-Hodgkin's >> Hodgkin's; 7-14% of non_hodgkin patients develop renal lymphoma; Diffuse infiltration in ~20% of cases; May manifest only as renal enlargement; Most common appearance is multiple low-density masses (commonly bilateral, ~1-3 cm in size); May appear as peri-renal soft tissue masses (surrounds but doesn't invade the kidneys); Gerota's Fascia (the fascia around the kidney) may appear thickened; Biopsy may be necessary
G-6-P Dehydrogenase
An enzyme in the hexose monophosphate shunt that reduces NADP to NADPH. NADPH subsequently provides the reducing power that converts oxidized glutathione to reduced glutathione, which in turn protects the RBC and its membrane from endogenous and exogenous oxidants. Deficiency in G6PD leads to hemolysis
What is vWF?
Von Willebrand Factor, deficiency is Von Willebrand dx
Two chemo drugs with cardiac toxicity
Doxorubicin and Epirubicin
Protein S.
Protein S is also a vitamin K–dependent protein which serves as a cofactor for activated protein C. Protein S deficiency may result in thrombotic disease. Hereditary deficiency of protein C or protein S is transmitted in an autosomal dominant fashion
Term used to describe changes in the bone marrow that are morphologically similar to, yet etiologically different from, megablastic change.
Failure of an organ or tissue to develop normally.
pathological conditions associated with too much cholesterol on the RBC membrane
liver disease, lipid disorders, chronic hemolitic anemia
lymphocytic leukemia
Most common type in young children, 2-4 years may affect some adults over 65-immautre B (85%) or T pymphocytes
Which type of anemia is characterized by the presence of ringed sideroblasts in the bone marrow?
sideroblastic anemia
What blood cell?
Involved in type I hypersensitivity
Mast cell
aplastic anemia etiology
most common is idiopathic; radiation, benzene, NSAIDs, chloranphenicol, alcohol, alkylating agents; infections: hepatitis, HIV, CMV, EBV, ParvoB19;
Describe drepanocytes.
Dark red to salmon color. Elongated cell with point on each end; may be curved or S-shaped. Aka sickle cell.
What are the functions of monocytes?
Killing of some microbes
Antigen presentation
Acute Promyelocytic Leukemia (M3)
AML with recurrent cytogenetic changes

Differentiation block is at promyelocyte stage
5-8% of AML
Most are t (15;17) -- 95%
creating PML/RARalpha fusion
others are also RARalpha mutants

Large granules with Auer rods
Extremely prone to clotting

Responsive to all-trans retinoic acid
Prognostic scale in MDS
Number of basts
Number of cytopenias

Worst - 5 months median survival, best -- 5 years median survival
Corrected WBC count
(uncorrected WBC#)/(100 + number of nucleated RBCs per 100 WBCs) X 100
What is the least abundant protein in plasma?
when might hgb be increased?
in polycythemia or dehydration
An abnormal proliferation of dysfunctional B lymphocytes is commonly associated with:
CLL Infectious Mono.
lymphocytes 25%
monocytes  6%
what two types of leukocytes are agranular?
What are their percentages? 
Which intermediate in heme synthesis is excreted in feces if in excess, and which steps does it precede/succeed?
coproporphyrinogen, comes before synthesis of protoporphyrinogen IX and after formation of uroporphyrinogen
Treatment of Mild Hemolysis
Supportive; Make sure the patient doesn't go into crisis and treat them if they do
MIXED lymph node hyperplasia
Granulomas Distinct type of immune reaction Infectious – bacteria (ie. TB) Non infectious – autoimmune (ie. sarcoid) Localized aggregates of histiocytes with suppuration: necrosis and neutrophils ie. cat scratch disease without suppuration
What supplentation should be given to pts w/ hereditary spherocytosis? Tx for aplastic crisis?
Folate. Transfusion.
basophils make up what percent of the diff
1 percent
reticulocyte count
The proportion (%) of RBC's in a specimen of blood that are immature reticulocytes Normal Range = 0.5% - 1.5%
Percentage of a given volume of blood that is occupied by erthryocytes

detects hemorrhage, polycythemia, erthyrocytosis, anemias, leukemia
Hematocrit determination
What is the growth factor for Erythrocytes?
IL-3 and Erythropoietin
Leukoerythrobastic reaction
Early forms of white and red blood cells in periphery, nucleated RBCs, tear drops

Seen with myelophthysic (marrow infiltration) and myelofibrosis
Most common cause of bleeding due to vit K deficiency
Over anti-coagulation
Anemia General Symptoms of Anemia
Integumentary Manifestations
↓ Hemoglobin
↓ Blood flow to the skin
↑ Concentration of serum bilirubin
↑ Serum and skin bile salt concentrations
What is an interferon
Something that body cells release during inflammation that aids leukocytes and macrophages to prevent entry into the body by a virus or antigen
Dysplastic changes of platelets frequently seen in MDS include?
Thrombocytopenia, abnormal granules, micromegakaryocytes
Gamma irradiation PRBCs
inactivates donor T cells and thus decreases risk of graft versus host disease; causes an increase in the rate of leakage of potassium out of RBCs during storage
what lab finding may indicate a pt has DHTR (delayed hemolytic transfusion reaction)?
Dec. Hb
What med. is rec. to be avoided in all d/o of the coagulation cascade?
Hodgkin's Disease (HD) v. Non-Hodgkin's Lymphoma (NHL)
Nodal Involvement and Spread - contiguous in HD; Extranodal involvement - can be entirely extranodal in NHL; Type of Infiltrate - HD has benign inflammatory cells present in response to the malignant Reed-Sternberg cells, while NHL has malignant cells themselves replacing nodes;Reed-Sternberg Cells - HD only, some NHLs have Reed-Sternberg-Like cells though;Age Distribution - Bimodal in HD
AML Prognosis
AML is dfficult to treat; There is a 60% remission rate with conventional chemotherapy, but only a 15-30% cure rate (i.e. disease free for 5 years) in that 60%; Tx may involve allogenic marrow transplantation
Deficiency of B12 results in what?
Trapping of THF as 5'mTHF
This test measures unbound Ab's in the serum
Indirect Coomb's Test
large amount of protein in urin is what?
bence jones protein
What are two other names for aplastic anemia?
hypoplastic anemia
pancytopenic anemia
chronic myelogenous leukemia treatment
imatinib is an inhibitor of the tyrosine kinase produced by disease
90% hematologic response and 60-70% loose Philadephia chromosome
if imatinib doesn’t work --&gt; bone marrow transplant
What are the congenital causes of qualitative platelet disorders?
1. Glanzmann's thrombaesthenia
2. Bernard-Soulier
Delayed hemolytic transfusion reactions
Occur 5-14 days later
Either a primary response to antigen or amnestic response with a latent population of memory cells

Fever, chills, signs of extravascular hemolysis
New alloantibody seen in serum

Rarely needs treatment
5q minus in MDS prognostics
Good prognosis, lower risk of progression to AML
Where does lymphocytopoiesis take place after birth?
In the peripheral lymphoid tissues lymphnodes spleen and peyer's patches
nursing/collab care for a neutropenic person?
1. administer hematopoietic growth factrs (granulocyte colony stimulating factor and granulocyte-macrophages colony stimulating facto

2. positive pressure room or high efficiency air

3. acquire culture from various sites

4. abx within 1 hour of febrile episode
Fetal blood volume: term, preterm
term: ~80 ml/kg; preterm: ~100 ml/kg
Describe lymphocyte depleted type of HL. How common is it? Who does it most often affect?
Sheets of RS cells lacking inflammatory cells. Rare (
Importance of Testing for Methylmalonic Acid Levels in a person with Borderline B12 levels
Normal B12 levels are 200-1000; In someone with s/s of B12 deficiency but who has a technically normal B12 level (ex: 250), measuring methylmalonic acid levels may assist in differentiating if B12 deficiency exists (if it's increased, they have B12 deficiency, even if their B12 level is in the normal range)
B cell lymphomas express
Kappa or Lambda light chain but NOT both
Defferentiate b/t thalassemia and sickle-cell anemia.
Thalassemia - too few globins syn., Sickle - incorrectly fxn globin
What are some causes of disseminated intravascular coagulation (DIC)?
bacterial endotoxins (sepsis)= most common cause
other infections
low blood flow
blood transfusions
Describe Sea Blue Histocyte.
The sea blue histocyte is a macrophage 20-60 microns in diameter with an eccentric nucleus. The cytoplasm contains varying numbers of prominent blue-green granules. These cells are found in spleen, liver, and bone marrow.
Prolong aPTT with no risk of bleeding
Deficiencies in HMWK, pre-kalikrein, XII
What is an echinocyte
A crenated cell usally caused by EDTA has cog wheel appearance
Which mechanisms are usually associated wneutropenia?
Impaired release from bone marrow to the blood, increased destruction, decreased production by bone marrow
Describe lymphocyte rich type of HL.
variants of RS cells w/ polylobated nuclei (popcorn cells), classic RS cells absent
What 2 Molecules stand in the way of Fibrinolysis?
Alpha-1-AntiPlasmin (acts against plasmin), and Plasminogen Activator Inhibitor (PAI) which is an acute phase reactant that acts against tPA - inhibiting tPA stops the conversion of plasminogen to plasmin and without plasmin, clots are not dissolved
Condition caused by an abnormality of the hexose monophosphate shunt
Cause of G-6-PD deficiency (inability to reduce glutathione)
The functional role of the cytoplasm
soluble portion of the cell where most of the cell's metabolic reactions take place
What is the second-line therapy for ITP and how often is it needed?
2/3 of pts relapse after steroid taper
What do T helper cells do?
Assist B cells in antigen recognition and their differentiation into plasma cells and assist activation of cytotoxic T cells suppressor T cells
Describe the pathogenesis of HDFN.
Occurs when a mother is RhD (-) and sensitization to Rh D antigens (by feto-maternal transfusion during pregnancy) can result in the production of maternal IgG anti-RhD antibodies. This becomes a problem in subsequent pregnancies when the baby is Rh-D (+)
Other than drugs, what else can cause thrombocytopenia d/t failure of platelet production?
Bone marrow failure, Etohism, aplastic anemia, autoimmune dz (RA, Lupus), viral infxns
The Intrinsic Pathway is just a test-tube artifact, what happens in vivo?
Factor 7 (of the extrinsic pathway) activates factor 9, which goes (via factor 8) on to activate factor 10 and then down the common pathway
Mechanism of chloroquine and related drugs
Causes a failure to inactivate heme or even enhanced toxicity of drug–heme complexes is thought to kill the parasites via oxidative damage to membranes.
if tablet shows up in urine when doing a shillings test, what does this indicate?
does not have pernicious anemaia because they are able to absorb it.
What is the treatment for IDA?
Ferrous sulfate 325 mg TID on empty stomach. May need to be given with food to decrease GI upset
In addition to iron ingestion, over 90 of iron in adults that is used for erythropoiesis is obtained through?
By products of red cell breakdown
From what causes of anemia would you see a low reticulocyte count?
with bone marrow problems and iron deficiency
There are 2 major etiologies for DIC
a. DIC results from exposure of tissue factor to circulating blood. b. DIC may also result from conditions leading to endothelial cell injury and/or Factor XII activation. There is a common association of gram–negative septicemia with DIC.
what to do if you suspect an anaphylactic reaction
stop blood, keep ns running, Code, cpr, maintain airway, give epinephrine
The best corrective action to take for a patient who exhibits platelet satellitism in their peripheral blood smear is:
Collect in tube wsodium citrate light blue top tube
How Many Mutations of the HIV Virus are Possible Per Day? What are the implications of this?
30,000 - given the propensity of the virus to mutate you need to use multiple drugs in different categories to treat patients with HIV so that the virus doesn't develop resistance to the drugs
What is a memory B cell
a B cell that is exposed to teh same antigen to form a line of plasma cells to attack that antigen
Name 4 social hxs that may result in folate def.?
1) Poor or fad diet 2) Malabsorption 3) Etohism 4) Pregnancy
Hairy-cell Leukemia
TRAP Positive
Seen with hyperleukocytosis (WBC>100,000); More typically seen in AML than ALL; Includes blurred vision, cerebrovascular dysfunction, or respiratory distress
Antiphospholipid antibody interferes with what?
Variation in RBC shape
Chediak Hegashi Syndrome
Betaglucocerebrosidase deficiency
large lysosome granules/dohle bodies/partial albinism
abnormal size of RBCs
hemo- / hemato-
pertaining to blood
What is thrombocytopathia?
abnormal platelet FUNCTION
Adult T cell lymphoma/leukemia associated w/
reverse heprin with what?
protamine sulfate
Gaucher's Disease
lipid storage disease
(large monocytes)
Endocrine causes of anemia
Low testosterone
Autoimmune thrombocytopenia
like ITP
Autoantibodies against platelets

Self-limited in kids with viral infections

Chronic and requiring immune suppression in adults
Thrombocytopenia Safety
Bedrest(platelet count < 20,000/mm3)
hemoglobin H
pitted golf ball apperance
Who does polycythemia typically affect?
50-80yo, men>women
Hemophilia A
Sex-linked recessive; Factor 8 Deficiency; Accounts for 80% of all Hemophilia cases; Can be mild (6-30% of normal levels of factor 8) which may be asymptomatic, to severe (less than 1% of the normal amount of Factor 8) in which case patients may bleed spontaneously.
Cloverleaf/Flower Like lymphocytes
Adult T cell Lymphoma/leukemia
Bendamustine hydrochloride
Mechanism: bifunctional agent – both alkylator and purine-like (antimetabolite) Uses: CLL and NHL Toxicity: DLT –hematopoietic toxicity(myelosuppression, etc.), GItoxicity, CNS toxicity (fever,chills, fatigue) Resistance: maybe onlypartical cross resistance withother alkylators
Ingestion and digestion of bacteria and particles by phagocytes.
The normal temperature of blood is
What blood cell?
Bilobed nucleus
Basophils &amp; eosinophils
RBC's of normal size (Normal MCV)
Eating weird stuff
Symptom of iron deficiency
amylophagia - starch
B12 deficiency
Decreased oral intake (vegans, alcohol)
Decreased absorption
deficiency in intrinsic factor
pancreatic insufficiency
gastrectomy, ileal resection
crohn's disease
neomycin, metformin, proton pump inhibitors
Nitrogen mustard
Used in CLL (in combos)
Neutropenia Clinical Manifestations
Low grade fever
What are T-Lymphocytes Responsible for?
Cell-mediated immunity
The red cell morphology associated with MMM is:
basophils 1%
neutrophils 65%
eosinophils 3% 
what three leukocytes are granular?
Thrombocytopenia and microangiopathic hemolytic anemia (elevated indirect bilirubin+reduced serum haptoglobin) should include what conditions in the differential?
Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome (TTP-HUS)
WHO AML Classification
AML with Recurrent Cytogenic Abnormaliies - AML with t(8;21), AML with t(15;17), AML with inversion (16); AML with t(11q23); AML not otherwise specified (NOS) - categoriezed based on degree of differentiation and cell type affected;NOTE: t(8;21), t(15;17) and inv (16) have a good prognosis, whereas t(11q23) has a bad prognosis
Etiologies of B12 Deficiency
Dietary deficiency (strict vegetarians); Impaired absorption (intrinsic factor deficiency - pernicious anemia, gastrectomy - Malabsorption); Competitive uptage (ex: parasites); Increased requirement (pregnancy)
Tx for sideroblastic anemia
pyridoxine in large doses
Cytologic Features Distinguishing Reactive Follicular Hyperplasia and Follicular NHL
Reactive Follicular Hyperplasia:Cells: Polymorphic in germinal centerMitotic act: Moderate to pronouncedPhagocytic act: prominentIntrafollicular area: Reactive cells.Follicular NHL:Cells: monomorphic in follicleMitotic act: lowPhagocytic act: lowintrafollicular area: Neoplastic cells
PMN (Polymorphonuclear Neutrophil)
A mature granulocyte (with neutrophilic granules) and a segmented nucleus (also called a segmented neutrophil). See Neutrophil.
multiple myeloma is cancer of what?
plasma cell
formed when ferrous iron (++) bound to heme is oxidized to (+++). Normal in 1% of circulating bloodreversible with strong reducing agents.
Thalasemia - \"mediteranean anemia\"
Abnormal (reduced) hemoglobin synthesis Manifestations of anemia Impairment of bone growth due to increased hematopoiesis Chronic transfusions A
What blood cell?
Polymorphonuclear leukocyte
Mainly describes Neutrophils
Also technically includes basophils &amp; eosinophils
multiple myeloma diagnosis
normo normo anemia, hypercalcemia, ↑BUN, ↑creatinine
lytic bone lessions on x-rays
Bence Jones protein
↑serum microglobulin B2 (75%)
electrophoresis with monoclonal IgG spikes
cofirmation --&gt; bone marrow biopsy showing &gt;10%plasma cells confirms
RBC's that have decreased amounts of hemoglobin and appear pale, and washed out on smear. (corresponds to a low MCH and low MCHC)
A dimorphic population of erythrocytes would be associated with...
-myelodysplastic syndromes
-vitamin B12, folate or iorn deficiencies - early in treatment process
MDS counts
Hypocelluarlarity in periphery
can be anemia, bicytopenia, pancytopenia

Hypercellularlity in bone marrow
Toxocaria canis
Human infection by roundworms of dogs

Dogs as pets suggestive of this potential cause of eosinophilia
Aplastic anemia treatment
Remove cause (stop radiation, meds)
Anti-thymocyte or antilymphocyte globulin
Cylcosporin, cyclophosphamide
Stem cell transplant
Solid phase antibody screen
Looking for non-ABO antibodies

Antigens on plate well
Anti-Ig tagged antibody
Sessile bodies
little nuclear protrusions; often with toxic granulation; doesn't show anything in itself
Diagnostic Studies
Laboratory data do not adequately assess RBC problems for 2 to 3 days
patho of thalassemia?
marrow is constantly producing immature RBC from oxygen deficit therefore there is chronic bone marrow hyperplasia
Hypogammaglobulinemia is a significant feature in half of patients with CLL. This complication can lead to?
Autoimmune hemolytic anemia
a clot that has broken off and migrated to another location in the circulatory system
Fibrin in the presence of Ca2+ activates ____ to cross link fibrin, which stabilizes and strengthens the clot.
Factor XIII
What is the form that most bilirubin is in?
Sickle Cell osteomyelitis caused by
S. aureus and Salmonella
Von Willebrands disease
Autosomal dominant. Lacks vWfactor which is the carrier protein for factor VIII. von Willebrand factor is a plasma glycoprotein that is necessary for adhesion of platelets to subendothelial connective tissue. The manifestations of this disease differ from hemo-philia in that bleeding is predominantly into the skin and mucous membranes (epistaxis, bruising, and menorrhagia). The pattern of bleeding resembles that seen in platelet disorders, rather than coagulation disorders. In contrast to hemophilia, hemarthrosis is rare in vWD patients.
The liver secretes bilirubin into the ________ as _______.
intestines, bile
Aplastic Anemia
Anemia caused by aplasia of bone marrow or its destruction by chemical agents or physical factors.
pernicious anemia is evident by what test
shillings test.
Basophilic Stippling
Consists of fine, diffuse to course, irregular granules of aggregated ribosomes and polyribosomesWright's Stain-dark blue to black Prominent in fetal and neonatal blood and exposure to lead and other toxic metals
What are some symptoms of posthemorrhagic anemia?
severe shock
lactic acidosis
Severe Congenital Neutropenia
a.k.a. Kostmann Disease
defect in neutrophil elastase (more severe defect than in Cyclic Neutropenia)
disseminated intravascular coagulation presentation
factor-type and platelet-type bleeding at any site
hemolysis can lead to acute renal failure, jaundice, confusion
results of increased intravascular hemolysis
increased indirect bilirubin
depleted haptoglobin and hemopexin
increased LDH
increased retic count
measurable methemalbumin
hyperplastic bone marrow
Describe megaloblastic anemia in PB.
Pancytopenia, oval macrocytes, Howell-Jolly bodies, nucleated erythrocytes, basophilic stippling, hypersegmentation of neutrophils, giant platelets, codocytes, schizocytes, spherocytes, dacryocytes.
T cell lymphomas
Less common
More likely to be extranodal
With nodal involvement are usually bad, and not as responsive to therapy as B cell
Follicular lymphoma epi
Most common of indolent lymphomas
22% of NH lymphomas

Disease of adults &gt;40
Typically presents as stage III or IV
Leukemia Clinical Manifestations
Relate to problems caused by
Bone marrow failure
Overcrowding by abnormal cells
Inadequate production of normal marrow elements
Anemia, thrombocytopenia, neutropenia
if neutropenic person is taking aminoglycosides, what should you watch out for?
nephro and ototoxicity
The RBC inclusions that are composed of denatured hemoglobin are called?
Heinz Bodies
Dissociation curve
HgbF shifts curve to left; preterm infants have a higher concentration of hgb F, higher affinity for O2, and lower pO2 at 50% saturation
What is a T lymphocyte def. that begins in fetal dev., but is not genetic?
DiGeorge syndrome
Reactive Leukocytosis
An excess of WBCs seen in inflammatory conditions; NEUTROPHILIA - acute inflammation, pyogenic infections, tissue necrosis; EOSINOPHILIA - allergies, parasites, drug reactions; MONOCYTOSIS - chronic infections; LYMPHOCYTOSIS - chronic and viral infections
What initiates a thrombosis in BV?
Endothelial injury exposes collagen
When would you give cryoprecipitate?
Give cryoprecipitate for coagulation disorders involving factor VIII or fibrinogen
An excess of red blood cells in the peripheral blood. Polycythemia vera: A chronic life-shortening myeloproliferative disorder involving all bone marrow elements, characterized by an increase in red blood cell mass and hemoglobin concentration.
increase in size of the cell that can lead to increase in organ or tissue size
Amount of hemoglobin in each erythrocyte (by weight)

Detects anemias, hereditary spherocytosis
Mean corpuscle hemoglobin (MCH)
What molecule becomes built up if you are deficient of B12?
Methylmalonic acid
cold agglutinin anemia diagnosis and treatment
normocytic anemia, reticulocytosis, ↑LDH, ↓haptoglobin, ↑indirect bilirubin, often spherocytes
Coombs test is specific
mild disease does not require specific treatment
steroids or splenectomy won't work
managed by avoiding cold weather or with azathiorpine, cyclosporine, cyclophosphamide, rituximab (anti-CD20 Ab)
What disease states are microcytes associated with?
-iron deficiency anemia
-sideroblastic anemia
-thalassemia minor
-chronic disease (occasionally)
-leasd poisoning
-hemoglobinopathies (some)
Explain antibodies in delay hemolytic transfusion reaction
Non intially
With transfusion, antibodies made
Antibodies attach to RBCs (direct Coombs positive)
More antibodies made, cells begin to lyse (direct and indirect positive)
All cells destroyed, antibodies in blood (indirect positive, direct negative)
Role of endothelium in steady state
Intact endothelium is antithrombotic

Prostacylin and NO secreting keeps plts from aggregating
Macrophages in lymph node
Main cell of medullary sinus
Abundant pale cytoplasm, oval nucleus, one nucleolus

A few in germinal center
Bone marrow sites (adults)
upper sternum; posterior illiac crest; lumbar vertebrae (NOT long bones)
Which two cells are azurophilic
Platelets and howell Jolly bodies
The blast count in most individuals with MDS is:
Less than 20
Mean corpuscular hemoglobin (MCH)
average weight of erythrocytes; number is inversely proportional to gestational age
Describe the expected finding in bone marrow of a pt w/ polycythemia vera.
Panhyperplasia (erythroid, myeloid, megakarocytic)
Normal Adult Hemoglobin
A tetramer of 4 globin chains;96% of Hg is HgA (2 alpha chains, 2 beta); 3% is HgA2 (2 alpha chains, 2 delta); 1% is HgF (fetal hemoglobin, 2 alpha chains, 2 gamma)
Acute Chest Syndrome
Seen in 30-50% of patients with Sickle Cell Disease; Clinical S/S include chest pain, new infiltrate on chest x-ray and fever; Most frequent cause of death in adults with SCD; Etiology is thought to be vaso-occlusion, but infarction, embolism, and pneumonia need to be considered
Total HIV Cases Worldwide
34-46 million adults and 5 million children
What are the clotting factors of the intrinsic pathway we must know?
Who should you use FFP for?
Replacement of multiple factor deficiencies in bleeding patients or in preparation for a surgical procedure Examples include liver disease, DIC, massive transfusion. Not for hemophilia or vW disease.
Name 3 types of erythroblasts.
1) Basophilic 2) Polychromatic 3) Acidophilic erythroblast
with polycythemia there is an increase in?
massive rbc's, excessive luekocytes, and overproduction of thrombocytes.
What kind of hemolysis do you get w/ IgG autoimmune hemolytic anemia?
Extravascular hemolysis
Anaplastic large cell lymphoma
T cell lymphoma
Can have a 2;5 translocation with ALK gene
ALK gene is a positive prognostic
A plasma ferritin of less than _______ reliably indicated IDA.
&lt; 30 micrograms/L
What do B-Lymphocytes differentiate into and do?
Differentiate into plasma cells and secrete antibodies
Difference between anisocytosis and poikilocytosis
aniso- size variation poikilo- shape variation
6 s/sx of Graft vs. Host Disease (GVHD)?
Erythroderma, maculopapular rash, fever, V/D, pancytopenia
What are the requirements for hemoglobin to function as an exchange medium between O2 and CO2?
capable of transporting large amounts of O2
Highly soluble
Take up and release O2 at appropriate pressures
be a good buffer
Most Common Causes of Anemia
Acute Blood Loss (GI bleed, traumatic bleed, AAA rupture), Iron Defficiency (subacute), Chronic Disease (renal, liver, infection, inflammatory, granulomatous, collagen vascular, and others); These 3 things (Acute blood loss, iron deficiency, and chronic disease states) account for about 75% of all anemias
Caused by factor VIII deficiency
Hemophilia A (most common hemophilia. X-linked recessive)
The functional role of ribosomes
active protein synthesis, in RBC site of hemoglobin synthesis
The enzyme deficiency in this cell causes an accumulation of what?
The deficiency in sphingomyelinase leads to the accumulation of sphingomyelin.
Signs and symptoms of acute immune hemolysis
Can be asymptomatic
Fever, chills
Pain at iv site
Red urine
Back (kidney) pain
What causes an increased MCHC?
falsely elevated = turbidity of sample or cold agglutinins; truly elevated - spherocytosis
what is folic acid deficiency caused by? (4)
think BARS
1. bad nutrition
2. alcoholism
3. renal dialysis
4. sm bowel d/o (malabsorption)
what is the physiologic fxn of platelets?
formation of primary hemostatic plug and provide surface for fibrin to form
What factors are involved in the common pathway?
I (fribrinogen), II (prothrombin), V, X, and XIII
1/3, 1/3, 1/3 Rule
A "rule" to help determine if a cell is normo-, hypo- or hyperchromic; In a normochromic RBC, the outer 1/3 should be red, the middle 1/3 should be pale (zone of pallor), and the other outer 1/3 should be red
When would you give platelets to someone?
Give this blood product for significant bleeding related to thrombocytopenia or a platelet defect (note that platelets lack Rh Ag).
what prophylactic measures should be done in pts w/ sickle cell anemia?
1) Pneumococcal pna vaccine (b/f 2yo and booster 3-5yrs later) 2) Hep B vaccination 3) H influ. vaccination 4) Penicillin prophylaxis for strep pneumo
What is an indirect Coombs' test?
Normal RBC's are added to patient's serum to determine if anti-RBC surface Ig's are present in the serum
Reticulocytes contain what? How do they appear?
contain meshwork of rRNA (this only stains with new Methylene blue); appear blueish and larger than normal RBCs
what is the hematocrit value compared to the hgb?
3x bigger than the hgb value
What is the etiology of NHL?
Unknown - poss viral cause (T cell leukemia-lymphoma virus, EBV, HIV)
What is the normal reticulocyte count and how do we determine it?
1% of erythrocytes in circulation and we stain with supravital stain to obtain the reticulocyte count
Silent Carrier Form of Alpha Thalassemia
Deletion of a single Alpha globin gene (___, Alpha/ Alpha, Alpha); Asymptomatic, Difficult to diagnose in adulthood
What are the dermatologic signs of B12 and folate def?
blotchy brown skin pigmentation of nail beds and skin creases
Calculating absolute values for cells
total WBC count x % of cell
EX: WBC Count of 8.2 x 10 3rd/μl
73 Neutrophils of 100 cells
so .73 * 8200=5.98 x 10 3rd/μl
What is the humoral Immune Response based on?
B cells secreting antibodies with the help of The Apc cell and T helper cell which activates into a plasma cell or memory cell.
Name 4 conditions that may be caused by lymph node masses in NHL?
1) Lymphedema 2) Uretal obs. 3) Vascular obs. (SVC syndrome, thrombophlebitis) 4) Epidural cord compression
If you're checking folate, what else should you automatically check?
B12 - because if B12 deficiency is really the problem, it may be masked by administering folate (i.e. the s/s of anemia will improve), but since you're not treating the real problem, you can still develop the demyelination
What occurs in anemic pts w/ CAD?
onset or exac. of angina or may precipitate an MI
What should you do when your slide does not match the Coulter print out?
check patient history; re run the sample on the Coulter; check for clots; verify it's the right slide; check for smudge cells (could be lymphs); call for a redraw;
What lab findings in a pt w/ thrombocytopenia indicate the cause is d/t ineffective production?
Variable size in smear, normal or inc. megakaryocyte
What drugs should be avoided in pts w/ G6PD deficiency? (7)
Asprin, Vit C and K, anti-malaria, Chlorophenicol, Procainamide, Quinidine, Sulfa-drugs
What is the routine tx for vWD?
No routine tx. Can give DDAVP if mild or vWF concentrate for very low levels. Can also give Factor VIII for rapid correction.
What is the most severe type of ABO incompatibility resulting in AHTR?
Type A RBCs given to pt w/ type O blood
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